Quality of life in patients with Stiff-Person Syndrome: Evaluation congruent with patient values using the schedule-direct weighting method

doi:10.21203/rs.3.rs-2995966/v1

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Quality of life in patients with Stiff-Person Syndrome: Evaluation congruent with patient values using the schedule-direct weighting method

https://doi.org/10.21203/rs.3.rs-2995966/v1

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The health-related quality of life (HRQoL) of patients with chronic progressive disease is low; however, various compensation mechanisms (medical, informal support, formal support, and environmental mechanisms) can improve and restore quality of life (QoL) even in cases of severe physical disability. In this study, we evaluate the QoL according to patient value by individual QoL assessment and identify the determinants of QoL of patients with Stiff-Person Syndrome (SPS) to consider specific support measures. As of December 2020, 13 patients diagnosed with SPS participated in a mixed study that included questionnaires and semi-structured interviews. The main positive factors cited by patients included ‘family support’ and ‘parental care’, whereas negative factors included ‘physical symptoms’ and ‘relationship with doctors’. Comparing SPS with other diseases revealed ‘parental care’ as a unique determinant of QoL in patients with SPS. ‘Parental care’ was identified as the most important area with the highest weight but low satisfaction; hence, in each case, support is needed to ensure that the patient’s caregiver was satisfactory. Additionally, ‘physical symptoms’ was the strongest negative area and can be alleviated by medical assistance. Furthermore, rehabilitation should address the relief of pain caused by muscle spasms and rigidity.

Biological sciences/Psychology

Health sciences/Diseases

Health sciences/Signs and symptoms

Stiff-Person Syndrome (SPS) is ‘a very rare autoimmune neuropathy characterised by painful muscle spasms and muscle rigidity’ [1, 2] [Insert video 1 about here]. The disease comes in many forms [1], and due to the low level of awareness regarding the disease, it is commonly misdiagnosed as an orthopaedic disease or a mental illness [1, 3, 4].

Nakane et al. indicate that severe muscular rigidity leads to lumbar lordosis and impairs voluntary movements such as walking, and it is severe enough for a patient to be unable to move their body [1]. Thus, when convulsions or rigidity attacks occur, patients cannot move or complete their usual activities of daily living (ADLs). Crispo et al. report inpatient care for SPS in the United States with the aim of describing inpatient treatment and characterizing 30-day inpatient readings (n = 703); ‘other hereditary and degenerative nervous system conditions’ was the most frequently recorded group of primary reasons for index SPS hospitalization (29.5%), and other leading reasons for index hospitalization included ‘diabetes complications’ (5.1%), ‘septicemia’ (3.9%), ‘other nervous system disorders’ (3.7%), ‘spondylosis, intervertebral disc disorders, and other back problems’(2.4%), and ‘respiratory failure, insufficiency, and arrest’ (1.7%) [5]. Sudden death due to autonomic neuropathy has also been reported [5–7]. Patients’ health-related quality of life (HRQoL) has been clinically assessed in previous studies. For instance, a review by Ortiz et al. reports on each treatment and shows that they were effective for completing ADLs, reducing convulsion frequency, and weakening of muscle tone according to the Ashworth Scale although the evaluation methods differed for each treatment [2]. Dalakas et al. evaluate the degree of stiffness, stiffness distribution, increased sensitivity (twitching and falling), and timed activity, and although there were individual differences/improvement in all items measuring SPS, the symptoms gradually worsened [8]. Sarva et al. investigate the presence of clinical symptoms after classifying the disease type [9]. Abbatemarco et al. utilise the Numerical Assessment of Pain, Spasm Frequency Scale, Lower Limb Modification Ashworth Scale, and Timed 25-foot Gait Scale to find that intrathecal baclofen therapy was effective for medically refractory spasticity caused by SPS and helped reduce the dosage of oral medications [10]. Furthermore, the patients’ quality of life (QoL) improved, their gait was maintained, and patients noted a decrease in convulsive frequency. [10].

In Japan, the diagnostic criteria for SPS were created in 2018 and later upgraded to include diseases with confirmed diagnostic criteria; however, they remain at the epidemiological survey stage. Although SPS is certified as an intractable disease, it is not specified as a designated intractable disease in Japan; as a result, it does not receive sufficient social support, which significantly affects patients’ mental conditions, economic statuses, and physical symptoms. Additionally, because SPS is not a designated intractable disease, the mechanism to compensate for the decline in QoL has not been studied sufficiently. The HRQoL of patients with incurable diseases decreases with disease progression. To date, studies have not correlated needs with HRQoL from the patients’ perspectives [11]. HRQoL focuses on a disease’s functional aspects and symptoms and thereby, evaluates medical aspects. However, HRQoL decreases in patients with intractable diseases and who are receiving end-of-life care, and patients’ needs and sense of well-being are not easily identifiable [2]. As there is no disease-specific evaluation method for designated intractable diseases, such as SPS, it is necessary to evaluate QoL according to patients’ values.

The Schedule for the Evaluation of Individual Quality of Life-Direct Weighting (SEIQoL-DW) is an individual QoL assessment method recommended by the World Health Organization that directly weighs QoL domains in the QoL assessment of intractable diseases and palliative care [12].

We evaluated the QoL of patients from the results indicating importance in patients’ lives and examined the determinants of patient QoL from the defined areas. Furthermore, we compared SPS with other diseases, extracted the determining areas of QoL, and identified the aspects that require support. This study is the first SEIQoL-DW evaluation of SPS. We hypothesize that the SEIQoL-DW index will decrease if the required compensation mechanism is ineffective.

Analysis

The mean values of levels, weights, and CWs were calculated using Microsoft Excel from the definitions of critical areas. The associations between diseases were compared using the Tukey–Kramer method. Finally, we analysed all cases and verified the relationship between the SB and SEIQoL-DW.

Fifteen people volunteered to participate, but one could not participate due to family circumstances, and one could not be contacted. Thirteen patients were able to participate in the study, but one of them was in complete remission and was therefore excluded.

Table 1 shows the attributes of patients. All patients had a disability certificate, but only four had access to the community-based integrated care system.

Table 1

Demographic characteristics (N = 12) of three male and nine female patients
Characteristics	Grade	n
Disability certificate	1	5
	2	4
	3	3
Disability pension	1	6
	2	4
	3	1
	none	1
n: number.

[Insert Table 1 about here]

Efficacy of the SEIQoL-DW testing

The average length of the interview was 42.25 ± 18.67 minutes (range: 20–85 minutes). Eight patients reported good comprehension, four reported low/unknown comprehension, and four required supplementary explanations. Further, five of the patients reported that they were not experiencing fatigue/decreased motivation, five reported slight fatigue/decreased motivation, and two reported very high fatigue/decreased motivation. None of the patients dropped out.

The interview time was relatively long because the examiner had breathing problems and had to read the explanations while experiencing breathing difficulties. In addition, the examiner had to listen carefully because the patients often suffered from dysarthria and dyspnoea. Of the five patients with ‘slight fatigue/decreased motivation’, one was able to make pie charts on their own, one was fatigued from sitting, one was unwell, one said the interview was easier than housework, and one complained about difficulty in hearing due to audio problems. Of the two patients who answered ‘very high’ regarding fatigue and loss of motivation, one laughed about having the same, usual fatigue, and the other said that he felt sick not because of the examination, but because of the medical condition.

Therefore, given that there was no loss of motivation or dropout, we considered the validity of the evaluation sufficient.

Validation from the definitions

Table 2 shows the classification of definitions and the contents of the cues. By definition, ‘physical symptoms’ was listed as the most common area, followed by ‘family support’, ‘anxiety’, and ‘hobby’. ‘Physical symptoms’ included difficulty ‘shopping’ and ‘eating’. Often, patients hesitated to go out because although they wanted to see and walk more, they were afraid of getting sick; hence, these difficulties were defined as ‘physical symptoms’. Furthermore, it was reported that one could not eat properly at night without the support provided by helpers due to their ‘physical symptoms’.

Table 2

Classification of definitions and the contents of cue
Definition area	Contents of the patient-mentioned cue	n
Physical symptoms	Living, I cannot read in my physical condition, body pain and tension, body tenseness, cramping, breathing, being able to walk freely, my health, I cannot do housework anymore, my health, one could not eat properly at night, shopping	12
Family support	Family (as indicated by two individuals); husband (as indicated by two individuals), husband, children and family, family health, family (wife)	7
Anxiety	Anxiety about not knowing what kind of illness I have, anxiety about illness, anxiety about the future, hoping to be positive, anxiety about medication, be positive, future facility admissions	6
Hobby	Hobbies (as indicated by two individuals), boats, piano, enjoying hobbies	5
Economic issues	Money (as indicated by two individuals), life after retirement, financial problems	4
Relationship with doctors	Hospital, relationship of trust with hospitals and doctors, relationship of trust with hospitals (doctors), lack of hospitals where you can be examined	4
Supporter	Care manager, environmental adjustment, living in elderly housing, getting help	4
Work	Job, working, helping farmers, job	4
Parental care	Caring for parents, parents, taking care of parents, wanting to spend time with parents	4
Emotional support	Snuggling, maintaining a lifestyle, Buddhism	3
Elimination of anxiety	I place anxiety-relieving objects such as photos and gifts from friends around myself, I do things that allow me to immerse myself in them because I get anxious if I am not doing anything.	2
Outing	I want to go out, go out on my own	2
Symptomatic stabilisation	Keep the wave of symptoms	1
Change of pace	Change my mood by going shopping	1
Hope	Challenge	1
n: number.

[Insert Table 2 about here]

Regarding the patients’ relationships with their doctor, there were also opinions reflecting both extremes. One patient said they were treated as fraudulent but stated, ‘I think it was the limit for doctors at that time. However, now the doctors say they will do whatever they can; hence, I want to build a trusting relationship with them instead of being repulsed by them’. In contrast, one patient said,

Even after being diagnosed with SPS, I was forced to go back and forth between the psychiatry and neurology departments. Some doctors do not believe I have SPS because they do not have antibodies; even now, they do not recognise SPS. I was told that I would no longer be examined at my hospital, and I thought of dying by committing suicide. I obtained a second opinion, and I was treated after writing a stern letter declaring that I had SPS and not a mental illness. Opinions have diverged within the department, and I still feel that hospitalisation is difficult. Doctors say that SPS does not cause respiratory function symptoms; however, it can still cause difficulties in breathing. Hence, I feel that doctors do not have a sufficient understanding of SPS.

Determinants of QoL

Table 3 depicts the determinants of QoL with weights. A positive area had a large weight and high level, while a negative area had a large weight and low level.

Table 3

Comparison of definitions, levels, weights, and category weights
	Definition area	ཎ	Level mean ± SD	Weight % mean ± SD	CW mean ± SD
Negative factor	Physical symptoms	12	30.83 ± 29.99	25.08 ± 21.01	6.76 ± 9.1
	Anxiety	6	25 ± 32.71	10.5 ± 12.06	1.78 ± 1.78
	Hobby	5	43 ± 33.84	10.5 ± 5.7	4.95 ± 4
	Relationship with doctors	4	63.75 ± 27.54	17.75 ± 9.8	8.44 ± 6.74
	Supporter	4	30 ± 21.6	13 ± 6.78	7.28 ± 4.38
	Work	4	13.75 ± 24.28	10.75 ± 8.1	2.69 ± 4.89
Positive factor	Family support	7	88.57 ± 14.64	34.71 ± 17.09	29.74 ± 13.24
	Economic issues	4	72.5 ± 27.54	14.75 ± 4.11	10.6 ± 5.2
	Parental care	4	50 ± 35.59	41.25 ± 30.1	24.75 ± 23.77
n: number, CW: category weight, SD: standard deviation

[Insert Table 3 about here]

‘Family support’ was the most powerful area; informal support was important and sufficient for SPS, as cited for other diseases. Further, ‘parental care’ was important even with respect to mean age and considering the role of the patients themselves. ‘Parental care’ was not satisfactory as patients suffered from ‘physical symptoms’, and ‘economic issues’ were not given much importance despite its low satisfaction. Furthermore, ‘physical symptoms’ formed the strongest negative area. As mentioned earlier, the ‘relationship with doctors’ was extreme as the level of ‘relationship with doctors’ was high, but the CW was low. Prior to the issuance of diagnostic criteria, patients with SPS had problems with awareness and understanding their medical professionals.

‘Supporters (formal)’ had a CW mean of 7.28 because it had a low level and low weight. Although the community-based integrated care system had few users, there was a latent need for such a system. Further, ‘work’ was separated from ‘economic issues’ to include the social role. Patients who wanted to work were required to work full-time. Those who could not work long hours felt that their physical condition was unacceptable. They complained that the work environment was not suitably prepared due to a lack of understanding of people with intractable diseases in the workplace. It had the lowest level as well, and the CW average was the second lowest among all areas, at 2.69.

‘Hobby’ seemed to be a buffering element for anxiety, with a level of 43 and a CW mean of 4.95. Finally, ‘anxiety’ had the second lowest level, at 25, and the lowest CW, at 1.78.

Comparison of SPS with other diseases

In this study, the mean SEIQoL-DW index for SPS patients was 52.55 ± 24.44 (range: 6.75–90) with a 95% confidence interval of 52.55 ± 15.53. The areas (cues) identified as important for patient QoL were ‘physical symptoms’, ‘family support’, and ‘anxiety’. Table 4 compares SPS in this study to previously reported diseases.

For palliative care outpatients, including 35 patients in the anti-cancer treatment group and 33 patients in the palliative care group, ‘family support’ was the most important, and the SEIQoL-DW index was particularly high in the palliative care group [14]. In the anti-cancer treatment group, ‘cancer treatment’ was the second most extracted region, and patients who placed the most importance on cancer treatment had lower SEIQoL-DW values compared to those who placed the most importance on other areas [14].

Table 4

Comparison of Stiff-Person Syndrome with other diseases
Disease	ཎ	Mean ± SD (Min-Max)	Cue mentioned	Author
SPS	12	52.55 ± 24.44 (6.75-90)	Physical symptoms, family support, anxiety	Current study
Anti-cancer treatment group	35	61.5 ± 21.0	In both cases, the first is family, but in the anti-cancer treatment group, cancer treatment is second.	Sakashita et al. [15]
Palliative treatment group	33	74.4 ± 14.9		Sakashita et al. [15]
Advanced cancer	62	59.17 ± 8 (6–95)	Family, health, social activities, religion, friendship, contentment/happiness	Dympa et al. [14]
PD	15	74.6 ± 18.1	Family, friends, hobbies, Health, and economic activities	Takahashi et al. [16]
ALS	80	73.3 ± 22.8 (1–98)	Social support, depression, religiosity, related to economic status	Chio et al [17]
SPS: Stiff-Person Syndrome, PD: Parkinson’s disease, ALS: amyotrophic lateral sclerosis, n: number.

[Insert Table 4 about here]

In another study, among 62 patients with advanced cancer, the most commonly extracted areas were ‘family support’, ‘health’, ‘social activity’, ‘religion’, ‘friendship’, and ‘contentment/well-being’ [15]. Moreover, among 15 patients with Parkinson’s disease (PD), the most frequently extracted areas were ‘family support’, ‘friends’, ‘hobby’, ‘health’, and ‘economic status’, which were significantly correlated with ‘communication’ in the Parkinson’s Disease Questionnaire-39. To improve the QoL of patients with PD, it is important to support social networks and promote communication [16]. In another study, 80 patients with amyotrophic lateral sclerosis were associated with ‘social support’, ‘depression’, ‘religiosity’, and ‘economic status’ [17].

SPS was compared with other diseases using the Tukey–Kramer method. Although the lowest mean index was obtained, a significant difference (p < 0.05) was found between advanced cancer and PD alone. The SEIQoL-DW indices of these diseases did not differ significantly from the SPS index.

Extracted cues

The most commonly extracted cues were ‘physical symptoms’, ‘family support’, and ‘hobby’. However, from the weighting perspective, ‘parental care’, ‘family support’, and ‘physical symptoms’ were the most commonly extracted cues.

‘Physical symptoms’ were considered a major negative area of QoL. ‘Parental care’, which was not heard of in other diseases, was considered an inevitable problem due to the concept of familism in Japan (which affirms caring for parents within the family rather than outsourcing this care) [18] and the average age of patients. It is assumed that the level of ‘parental care’ reduced ‘physical symptoms’ according to the involvement of parents before disease onset. Finally, as for any other disease, we consider family to be an important aspect of emotional and financial support.

QoL determinants in patients with SPS from defined regions

The positive QoL determinants from definitions were ‘parental care’, ‘family support’, and ‘economic issues’, and the negative determinants were ‘physical symptoms’, ‘relationship with doctors’, ‘hobby’, ‘supporter’, ‘anxiety’, and ‘work’. The study indicated ‘family support’ as the strongest area. Furthermore, informal support was found to be important and adequately provided for SPS, as indicated when compared to other diseases. As the level of ‘parental care’ is lower than ‘family support’, the former requires some type of support, for example, one patient expressed a desire to take care of her parents herself.

Therefore, we believe it is necessary to secure individual means of transportation and adjust the living environment so that it is more accessible and user-friendly for patients (e.g. the adjustment of mobility and arrangements to ease housework at the parents home, such as lightweight vacuum cleaners, handrails, attachments for trolleys, and height-adjustable chairs in the kitchen), instead of simply recommending the introduction of a third party. If a problem can be solved by environmental considerations, we should provide support in the form of proposals. Hence, healthcare workers should consider all areas of patient activity and not just their home environment. Finally, the results suggest that ‘economic issues’ are unimportant, and ‘living with family’ and ‘receiving a disability pension’ likely have no impact on QoL.

‘Physical symptoms’ was the strongest negative area, of which medical assistance was an important part. In rehabilitation, only case reports are available. Furthermore, there are only a few cases where confounding areas can be ruled out; hence, researchers should consider this area for future studies.

‘Relationship with doctors’ was a high-level area; however, its CW was low because it was associated with negative experiences in some patients. This indicates the necessity of increasing the awareness of SPS. Unless it becomes a designated intractable disease, it cannot be accessed at the Intractable Disease Consultation Center. Hence, a definitive diagnosis is expected to take some time.

‘Anxiety’ had the lowest weight, despite being mentioned by half of the patients. In addition, ‘anxiety’ had the lowest level and CW. Patient statements revealed that anxiety develops with the progression of symptoms. Although ‘anxiety’ had a low weight, patients require psychological support during symptom progression.

A ‘job’ is a small pension for young patients with SPS. The financial burden posed by the child until they become independent is high. Most of the SEIQoL-DW participants are on grade 2 or higher disability pension. However, those with a grade 3 pension may find it tough to survive without earning some income. Such individuals have fluctuating physical conditions that are easily exacerbated by external and emotional stimuli. Voluntary movements induce muscle rigidity and muscle spasms, making it difficult for grade 3 pensioners to continue with their occupations. Many patients expressed wanting self-actualisation, and most of them were working. However, gaining what was lost in a new form is a major hurdle under the current system. Although some IT business sites practice preferential treatment for individuals with SPS, these individuals may find that the physical strength to work during office hours is difficult to maintain. In addition, even if possible to integrate into the workplace or environment, it is difficult to realise that such workers need preferential treatment unless there is a vocational training facility nearby to obtain the necessary qualifications (in principle, commuting is required under the national system). Although correspondence courses have already been implemented in some prefectures, they have not aided career advancement much due to the small number of courses available and their short duration. Practical career advancement requires an ever-increasing level of support. Moreover, patients with intractable diseases must cope with changes in their illness, which act as a barrier to career advancement.

Comparison of SPS with other diseases

Although patients with SPS in this study had lower SEIQoL-DW indices (52.55 ± 24.44 (range 6.75–90)) than other diseases, the SPS index was not significantly different from the indices of other diseases such as cancer, PD, and ALS. Interestingly, the area of ‘parental care’ listed in SPS is not found in other diseases. Furthermore, the study found that SPS patients seek supporters similar to those for ALS, which indicates that there are several barriers to the use of formal support. In the future, researchers should clarify the barriers and examine why they could not be overcome. The main limitation of this study is the small sample size due to SPS being a rare and intractable disease; therefore, the results cannot be generalised. As some patients have raised issues in the past, it is necessary to investigate the problems faced by them before obtaining a disability certificate.

New institutional support measures, such as a community-based comprehensive care system for intractable diseases that are not designated as intractable diseases are needed, and we hope that this study can make contributions in this regard.

The relationship between the SB and SEIQoL-DW indices was confirmed in areas where psychological adaptation was achieved (more than 45 points) (Fig. 1); well-being was observed in patients with more than 60 points, and the high SB group was polarized.

Four patients did not reach psychological adjustment, three reached psychological adjustment, and five were happy. Case 2 indicated a state of well-being paradox with all positive cues.

[Insert Fig. 1 here].

This study demonstrated that an important area of SEIQoL-DW for patients was physical symptoms due to pain, such as cramps and stiffness. The results clarified patient needs and examined possible countermeasures. From the rehabilitation perspective, it is necessary to adjust the environment to alleviate patients’ pain, enhance their social activities, and build support that enables young patients who wish to engage in economic activities to receive necessary vocational training. Institutional reform of social support is also required. Remuneration for care for the physically disabled was revised in the fiscal year of 2021; however, few business sites comply with it. As a result, patients with incurable diseases requiring more frequent involvement are not adequately covered by the current care plan regime [19]. Hence, it is necessary to separate intractable diseases from the Welfare Act for Persons with Disabilities. In addition, it is difficult to respond appropriately to intractable diseases that are not fully understood. Therefore, municipalities need to secure not only a small number of business establishments but also a wide variety of business sites regarding quality and quantity and strengthen the information provision of intractable disease consultation centres to enhance the understanding of intractable diseases.

Due to the relationship between SEIQoL-DW and SB, an SEIQoL-DW score of 60 points or more could be used to indicate well-being. Psychological adaptation was defined as a higher positive CW total score than the negative CW total score. Three criteria were used to indicate a failure to accomplish psychological adaptation: a total negative CW total score that is more than the total positive CW total score; a substantial positive CW total score and a significant number of negative cues; and a low level of the most important cue. The third crucial factor, the low level of the cue, clearly indicates the need to be more supportive of patients. Although it is critical to utilise the SEIQoL-DW to precisely define psychological states, in this study, the number of samples is small; therefore, it is necessary to secure more samples to improve the accuracy in the future. Furthermore, longitudinal evaluation should be considered to confirm changes in the content of support.

This is a cross-sectional observational study, in which participants were recruited through patient groups and Facebook and invited for interviews. The SEIQoL-DW was conducted in person from May 28 to June 18, 2021.

This study was performed in line with the principles of the Declaration of Helsinki. Approval was granted by the International University of Health and Welfare Ethical Review Board (10 Feb 2021/No. 20-Io-149). Written informed consent was obtained from all individual participants included in the study.

The SEIQoL-DW was completed on the interview record form online. It provided an overall assessment to the interviewer regarding response time, understanding, fatigue/reduced motivation, and adequacy of the data obtained. The SEIQoL-DW consisted of predetermined questions and explanations and provided supplementary explanations according to the level of understanding of each patient.

First, the interviewer asked, ‘What are the five most important areas of your life right now?’ The participants then named five areas they thought were currently making their daily lives enjoyable or sad, that is, the areas (cues) that determined their QoL. The definition of each cue is important to obtain from the patient, that is, the underlying meanings of their answers were defined by asking them to talk about it. For example, as part of the grounded theory, going to Church (cue) is defined differently depending on whether it is for religious reasons or socialising with friends [12]. Second, the five cues were levelled using the Visual Analog Scale. Finally, the weight of each cue was displayed by importance (using a pie chart). The category weight (CW) was calculated by multiplying the levels by the weights, and the SEIQoL-DW index was calculated as the sum of the five CWs. Moreover, cues with low CWs—despite being considered important by individuals—were the cues that required support, and those with high CWs were crucial for determining QoL. The range of the total index was 0–100. The higher the index, the better the QoL of patient attributes (disability certificate, disability pension, and the self-assessment Barthel Index (SB) [13], which were used for comparison with the SEIQoL-DW index.

Availability of data and materials

The data generated or analysed during the current study are partially available upon request from the corresponding author. These data may include age and gender and no other personal information, up to March 31, 2024. The research plan, which is in Japanese, is also available.

Acknowledgments

We express our sincere gratitude to all the patients who participated in the interview. We would like to thank Editage (www.editage.com) for English language editing.

Author contributions

Conceptualisation, writing–review, formal analysis, methodology, English translation, visualisation, and editing were performed by CK. Formal analytical guidance, methodological guidance, visualisation–original draft, writing–review guidance, and guidance were provided by YM. Conceptualisation guidance, methodology guidance, supervision, and writing–review were performed by YN.

Funding

This work was supported by the International Graduate School of Health and Welfare Student Research Fund.

Competing interests

The authors have no relevant financial or non-financial interests to disclose.

Consent for publication

The authors affirm that human research participants provided informed consent for the publication of their data in academic papers.

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No competing interests reported.

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Quality of life in patients with Stiff-Person Syndrome: Evaluation congruent with patient values using the schedule-direct weighting method

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Abstract

Figures

Introduction

Analysis

Results

Efficacy of the SEIQoL-DW testing

Validation from the definitions

Determinants of QoL

Comparison of SPS with other diseases

Discussion

Extracted cues

QoL determinants in patients with SPS from defined regions

Comparison of SPS with other diseases

Conclusion

Materials and methods

Declarations

References

Additional Declarations

Supplementary Files

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